ArticleMolecular neurobiology2016
Oxidative Stress, Disrupted Energy Metabolism, and Altered Signaling Pathways in Glutaryl-CoA Dehydrogenase Knockout Mice: Potential Implications of Quinolinic Acid Toxicity in the Neuropathology of Glutaric Acidemia Type I.
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Article in Molecular neurobiology, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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21citing papers in PubMed
3.2field-weighted citation impact, top 8% of its field
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21 citing papers in PubMed, 40 citations in OpenAlex.
- L-carnitine modulates molecular responses associated with inflammation, endoplasmic reticulum stress, and neurotrophic signaling in cerebral cortex of neonatal GcdhMetabolic brain disease · 2026Article
- L-Carnitine Prevents Lipopolysaccharide-Induced Inflammatory Response, Microglia Activation and Neuronal Damage in Cerebral Cortex, as Well as Neuromotor Development Delay in Neonatal Glutaryl-CoA Dehydrogenase Deficient (GcdhJournal of inherited metabolic disease · 2026Article
- Intracerebral Administration of Hydrogen Sulfide Impairs Bioenergetics, Redox Status and Mitochondrial Quality Control in Rat Striatum.Neurotoxicity research · 2025Article
- Acute effects of intracerebroventricular administration of α-ketoisocaproic acid in young rats on inflammatory parameters.Metabolic brain disease · 2023Article
- Tetrahydrobiopterin: Beyond Its Traditional Role as a Cofactor.Antioxidants (Basel, Switzerland) · 2023Review
- NRF2 mediates melanoma addiction to GCDH by modulating apoptotic signalling.Nature cell biology · 2022Article
- Disturbance of Mitochondrial Dynamics, Endoplasmic Reticulum-Mitochondria Crosstalk, Redox Homeostasis, and Inflammatory Response in the Brain of Glutaryl-CoA Dehydrogenase-Deficient Mice: Neuroprotective Effects of Bezafibrate.Molecular neurobiology · 2022Article
- An integrated cytokine and kynurenine network as the basis of neuroimmune communication.Frontiers in neuroscience · 2022Review
- Caenorhabditis elegans as a model for studies on quinolinic acid-induced NMDAR-dependent glutamatergic disorders.Brain research bulletin · 2021Article
- Nuclear Factor Erythroid-2-Related Factor 2 Signaling in the Neuropathophysiology of Inherited Metabolic Disorders.Frontiers in cellular neuroscience · 2021Review
- Lipopolysaccharide-Elicited Systemic Inflammation Induces Selective Vulnerability of Cerebral Cortex and Striatum of Developing Glutaryl-CoA Dehydrogenase Deficient (GcdhNeurotoxicity research · 2020Article
- Involvement of the Cholinergic Parameters and Glial Cells in Learning Delay Induced by Glutaric Acid: Protection by N-Acetylcysteine.Molecular neurobiology · 2019Article
- Neurological manifestations of organic acidurias.Nature reviews. Neurology · 2019Review
- Bezafibrate In Vivo Administration Prevents 3-Methylglutaric Acid-Induced Impairment of Redox Status, Mitochondrial Biogenesis, and Neural Injury in Brain of Developing Rats.Neurotoxicity research · 2019Article
- Mitochondrial energetics is impaired in very long-chain acyl-CoA dehydrogenase deficiency and can be rescued by treatment with mitochondria-targeted electron scavengers.Human molecular genetics · 2019Article
- Evidence that Thiosulfate Inhibits Creatine Kinase Activity in Rat Striatum via Thiol Group Oxidation.Neurotoxicity research · 2018Article
- Toxic Synergism Between Quinolinic Acid and Glutaric Acid in Neuronal Cells Is Mediated by Oxidative Stress: Insights to a New Toxic Model.Molecular neurobiology · 2018Article
- Evaluation of plasma biomarkers of inflammation in patients with maple syrup urine disease.Journal of inherited metabolic disease · 2018Article
- Hypoxanthine Induces Neuroenergetic Impairment and Cell Death in Striatum of Young Adult Wistar Rats.Molecular neurobiology · 2018Article
- Experimental Evidence that In Vivo Intracerebral Administration of L-2-Hydroxyglutaric Acid to Neonatal Rats Provokes Disruption of Redox Status and Histopathological Abnormalities in the Brain.Neurotoxicity research · 2018Article
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Authors and funding
8 authors at 3 institutions in 2 countries.
Bianca SeminottiDepartamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos N° 2600-Anexo, CEP 90035-003, Porto Alegre, RS, Brazil.
Alexandre Umpierrez AmaralDepartamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos N° 2600-Anexo, CEP 90035-003, Porto Alegre, RS, Brazil.
Rafael Teixeira RibeiroDepartamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos N° 2600-Anexo, CEP 90035-003, Porto Alegre, RS, Brazil.
Marília Danyelle Nunes RodriguesDepartamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos N° 2600-Anexo, CEP 90035-003, Porto Alegre, RS, Brazil.
Ana Laura Colín-GonzálezLaboratorio de Aminoácidos Excitadores, Instituto Nacional de Neurología y Neurocirugía, Manuel Velasco Suárez, SSA, México, DF, México.
Guilhian LeipnitzDepartamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos N° 2600-Anexo, CEP 90035-003, Porto Alegre, RS, Brazil.
Abel SantamaríaLaboratorio de Aminoácidos Excitadores, Instituto Nacional de Neurología y Neurocirugía, Manuel Velasco Suárez, SSA, México, DF, México.
Moacir WajnerDepartamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos N° 2600-Anexo, CEP 90035-003, Porto Alegre, RS, Brazil. mwajner@ufrgs.br.
Universidade Federal do Rio Grande do Sul · BRInstituto Nacional de Neurología y Neurocirugía · MXHospital de Clínicas de Porto Alegre · BR
Funding
No grant is acknowledged in the PubMed record.
Abstract
We investigated the effects of an acute intrastriatal QUIN administration on cellular redox and bioenergetics homeostasis, as well as on important signaling pathways in the striatum of wild-type (Gcdh
Indexed as
Amino Acid Metabolism, Inborn ErrorsAnimalsAntioxidantsBrain Diseases, MetabolicDietElectron Transport Complex IVEnergy MetabolismExtracellular Signal-Regulated MAP KinasesFluoresceinsGlutaryl-CoA DehydrogenaseGlutathioneGlutathione PeroxidaseLactatesLipid PeroxidationMalondialdehydeMice, Knockout2',7'-dichlorodihydrofluoresceinAntioxidantsElectron Transport Complex IVExtracellular Signal-Regulated MAP KinasesFluoresceinsGlutaryl-CoA DehydrogenaseGlutathioneGlutathione PeroxidaseLactatesMalondialdehydeNfe2l2 protein, mouseNF-E2-Related Factor 2NF-kappa BNitratesNitritesProto-Oncogene Proteins c-aktQuinolinic AcidSulfhydryl CompoundsSuperoxide DismutaseSuperoxide Dismutase 2Energy metabolismGlutaric acidemiaInflammatory responseQuinolinic acidRedox homeostasisSignaling pathways
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.